The scariest and most deadly form of Ehler-Danlos is the heart or vascular versions. Let me back up for a second and tell you a bit more about the fine details of this disorder. It’s easily described as a dysfunctional collagen issue. That’s the stuff that basically holds your whole body together like glue. It’s everywhere…skin, joints, organs, blood vessels, all of it. So when it’s faulty everything feels loose and unstable. For me, that shows up as joints that bend too far, pop out, sprain easily, and just generally refuse to stay where they’re supposed to be. It’s not like a cool party trick flexible (though I can perform many “tricks!”) it’s more like, “Oh great, my shoulder slipped out while reaching for the milk, again…” or, “I sat down and turned sideways slightly and caused my whole knee to come out of its socket.” Both examples are also true stories...
Pain is a permanent houseguest. My skin bruises easily and cuts, injuries, and surgeries take forever to heal. Migraines and stomach issues are also super common symptoms. It’s an invisible illness, and those of us with EDS have adopted the nickname “Zebras,” a term that comes from the phrase, “sometimes, when you hear hoofbeats, it really is a zebra.” This demonstrates how common it is for misdiagnoses when it comes to a random variety of symptoms. Each EDS patient is completely different and has their own individual difficulties and issues. This makes it hard to diagnose and especially hard to treat. There is no cure, the main goal is management. Physical therapy, braces, pain interventions, and using assistive devices and wheelchairs are all part of it.
I’ve quickly become a very capable ambulatory electric wheelchair user. It has been such a relief to have the freedom to use my “spoons” for the activities I choose and stick to the chair for everything else when I’m completely out of steam. I don’t have to spend every moment dreading having to get up and do something active. I can wheel back and forth across the house all day long with no energy loss whatsoever. I finally have some freedom back, and it has been beautiful.
The hardest part has been this balancing act of wanting to do normal life things like chase little Princess around or take our dog for a walk. I know that if I do those activities, I will be punished for it later. My choices are to push through and pay for it, or I can pace myself and feel like I’m being lazy and missing out. It’s an invisible disability, and it’s been even hard to accept myself. I’ve had to come to the realisation that I’ve been literally grieving the loss of a future I dreamed of but can no longer have. I am grieving the loss of self and abilities. It’s been a humbling and depressing process to navigate. I also battle with the heavy weight that looms constantly over me like a dark cloud raining reminders on me that I am a burden on those around me. My issues don't just affect me…they touch every single person in my life too and I hate that so very much.
It hasn’t been helpful at all that this is the worst state and location for mental health. Every good therapist or psychiatrist that had the abilities to, has abandoned this miserable state. This awful state that will literally prosecute those doctors who even HINT at reaffirming or treating any sort of gender dysphoria suffering patients even. It has taken me three years to find a therapist and doctor to see and treat me. They are pretty much burnt out and barely available… but I AM trying, okay? I am. My mental health is a major discussion all on its own. However, let's swing back to vascular EDS.
It’s scary stuff! You don’t want to be messing around with the heart or vascular system getting too flexible. I found a Cardiologist right away after that geneticist appointment. They said the ECG showed I had a minor arrhythmia and they did want to run a full CT Angiogram and echocardiogram following that too. The CT contrast was so painful!! It burned like lava for about ten seconds, and I had no idea to expect that one bit. The Echo was a simple ultrasound-like test with zero pain this time, thankfully. I’ll take any diagnostic test that involves zero pain over anything else please!
They found my heart to be mildly enlarged and a mildly leaky valve, but the aorta was perfect, and there are zero signs of aneurysms which would be the typical vEDS diagnostic criteria. After that, the doctor was totally done with me. He was like, “Our findings are pretty normal, and we can recheck in ten years.” I guess a mildly enlarged heart and minorly leaky valves are completely normal. And then he refused to listen to my other symptoms that are very Postural Orthostatic Tachycardia Syndrome heavy (POTS for short). Basically, I have super low blood pressure, always noted every single week by every single doctor I visit. When I stand up too quickly, my heart rate skyrockets, blood pressure drops, and I get very light headed and have tunnel vision. I’ve passed out a few times throughout my life just from standing up too quickly. The first time was at age eight, in front of my whole class! It makes doing things like standing still in a line a marathon, or getting up from a laying down position a very slow process. I get overheated very easily and am prone to heatstroke. I have to balance a heavy salt intake with lots of fluids for bonus hydration as well.
However, he did not want to listen... He wanted to TALK. He babbled on and on about how he doesn’t ever give out multiple diagnoses to people because he doesn’t believe it to be helpful in any way, how dumb orthopedic surgeons are, and that I have temperature issues because I must not dress appropriately. He said the worst thing to possibly wear is HALF SLEEVES. But those are my actual favorite cut of shirt because of the temperature regulation control they provide. He was nuts, and I cut him off after he was like, “I promise I’m treating you the same as I would my own sister! Har har har!”
I currently have a three-month wait to see a new cardiologist who I plan on getting a formal second opinion from and official POTS testing. I’ve also gained a bit of weight which is horribly abnormal for me. I’m only mildly worried its some form of edema, especially since it’s all mainly been gained in the lower part of my body. I also have a weird thing with reduced circulation in my hands and feet called Raynaud's Phenomenon. It makes my fingers and toes turn weird colors and feel numb/painful when triggered by cold or stress. I only need to make sure and double check on my heart before any more surgeries pop up.
Surgeries are a reality I need to prepare for being a lifelong occurrence as things break down and need to be put back together. My current avoidance is a left hip surgery and a double shoulder surgery I haven't even acknowledged in a year. Nowadays my left shoulder rarely stays in its socket. Subluxing is the term for when a joint partially dislocates. My right shoulder subluxes often but hasn’t come out all the way in a few months. And do I go to the doctor when this happens? No way. It happens so often, and they make me take multiple x-rays anytime I try to say I have something for them to look at. Like the last four x-rays don’t count. It’s not like you can even see the tendons and ligaments on an x-ray. If I broke a bone dislocating, I WOULD KNOW! My current freak skill is how well and effectively I can put my own shoulders back in their sockets when they fall out. Like the ragdoll Sally, from Nightmare Before Christmas, after she flings herself from her prison of a tower. I also have to wake up most mornings, fling myself out of bed, and put myself all back together. Piece. By. Piece…
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